Diagnosis and Management of Patients with Connective Tissue Disease-related Fibrosing Interstitial Lung Diseases.

Q3 Medicine

Open Respiratory Medicine Journal Pub Date : 2023-08-15 eCollection Date: 2023-01-01 DOI:10.2174/18743064-v17-e230714-2022-26

Bonnie Wang, Vivek Nagaraja

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Abstract

Background: Fibrotic interstitial lung disease is an important driver of morbidity and mortality in patients with connective tissue diseases (CTD). Due to the lack of prospective randomized trial data in this population, practice pattern variation exists in the management of patients with CTD.

Case presentation: This case series describes three patients, each with a different background of autoimmunity complicated by fibrotic interstitial lung disease (ILD). We review their initial presentations, follow their disease trajectories on currently available treatments, and reference forthcoming clinical trials.

Conclusion: Clinical impact or potential implications. Response to immunosuppression and antifibrotic therapy is variable in patients with connective tissue disease-related fibrosing interstitial lung disease. Data from prospective clinical trials and longitudinal registry studies will conceivably provide additional insight into improving care for these patients.

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结缔组织疾病相关纤维化间质性肺疾病患者的诊断和治疗。

背景：纤维间质性肺病是结缔组织疾病（CTD）患者发病率和死亡率的重要驱动因素。由于缺乏该人群的前瞻性随机试验数据，CTD患者的管理存在实践模式差异。病例介绍：本病例系列描述了三名患者，每个患者都有不同的自身免疫背景，并伴有纤维间质性肺病（ILD）。我们回顾了他们的初步陈述，跟踪了他们目前可用治疗方法的疾病轨迹，并参考了即将进行的临床试验。结论：临床影响或潜在影响。结缔组织疾病相关纤维化间质性肺病患者对免疫抑制和抗纤维化治疗的反应是可变的。来自前瞻性临床试验和纵向注册研究的数据将为改善这些患者的护理提供更多的见解。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Open Respiratory Medicine Journal Medicine-Pulmonary and Respiratory Medicine

CiteScore

1.70

自引率

0.00%

发文量

期刊介绍： The Open Respiratory Medicine Journal is an Open Access online journal, which publishes research articles, reviews/mini-reviews, letters and guest edited single topic issues in all important areas of experimental and clinical research in respiratory medicine. Topics covered include: -COPD- Occupational disorders, and the role of allergens and pollutants- Asthma- Allergy- Non-invasive ventilation- Therapeutic intervention- Lung cancer- Lung infections respiratory diseases- Therapeutic interventions- Adult and paediatric medicine- Cell biology. The Open Respiratory Medicine Journal, a peer reviewed journal, is an important and reliable source of current information on important recent developments in the field. The emphasis will be on publishing quality articles rapidly and making them freely available worldwide.